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Merck
모든 사진(3)

주요 문서

SAB1402110

Sigma-Aldrich

Monoclonal Anti-NR0B1, (C-terminal) antibody produced in mouse

clone 1F10, purified immunoglobulin, buffered aqueous solution

동의어(들):

AHC, AHCH, AHX, DAX-1, DAX1, DSS, GTD, HHG, NROB1

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About This Item

UNSPSC 코드:
12352203
NACRES:
NA.41

생물학적 소스

mouse

Quality Level

결합

unconjugated

항체 형태

purified immunoglobulin

항체 생산 유형

primary antibodies

클론

1F10, monoclonal

양식

buffered aqueous solution

분자량

antigen ~38.21 kDa

종 반응성

human

기술

capture ELISA: suitable
direct immunofluorescence: suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL

동형

IgG3κ

NCBI 수납 번호

UniProt 수납 번호

배송 상태

dry ice

저장 온도

−20°C

타겟 번역 후 변형

unmodified

유전자 정보

human ... NR0B1(190)

일반 설명

This gene encodes a protein that contains a DNA-binding domain. The encoded protein acts as a dominant-negative regulator of transcription which is mediated by the retinoic acid receptor. This protein also functions as an anti-testis gene by acting antagonistically to Sry. Mutations in this gene result in both X-linked congenital adrenal hypoplasia and hypogonadotropic hypogonadism. (provided by RefSeq)

면역원

NR0B1 (NP_000466, 361 a.a. ~ 470 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
IKCFLSKCWSLNISTKEYAYLKGTVLFNPDVPGLQCVKYIQGLQWGTQQILSEHTRMTHQGPHDRFIELNSTLFLLRFINANVIAELFFRPIIGTVSMDDMMLEMLCTKI

생화학적/생리학적 작용

The encoded orphan nuclear receptor is involved in the growth and activity of the adrenal and reproductive organs. It is significantly associated with the growth of gonads, development of sexual characteristics and steroidogenesis. The expression of NR0B1 (nuclear receptor subfamily 0 group B member 1) gene is regulated by Nanog (transcription factor). NR0B1 is known to control pluripotency and differentiation of embryonic stem cells in mice. Mutation in NR0B1 is importantly associated with X-linked adrenal hypoplasia congenita. Mutations in NR0B1 is also known to downregulate the androgen receptor activity, also leading to defect in spermatogenesis.

물리적 형태

Solution in phosphate buffered saline, pH 7.4

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Storage Class Code

10 - Combustible liquids

WGK

WGK 1

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable


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문서 라이브러리 방문

A Novel Mutation of DAX-1 Associated with Secretory Azoospermia.
Mou L
PLoS ONE, 10(7), 1-11 (2015)
X-linked adrenal hypoplasia congenita and hypogonadotropic hypogonadism: Identification and in vitro study of a novel small indel in the NR0B1 gene.
Yu T
Molecular Medicine Reports, 13(5), 4039-4045 (2016)
Isolated 46,XY gonadal dysgenesis in two sisters caused by a Xp21.2 interstitial duplication containing the DAX1 gene.
Barbaro M
The Journal of Clinical Endocrinology and Metabolism, 92(8), 3305-3313 (2007)
A novel DAX-1 mutation in two male siblings presenting with precocious puberty and late-onset hypogonadotropic hypogonadism.
Liu Y
Journal of Pediatric Endocrinology & Metabolism : JPEM, 30(3), 349-353 (2017)
Yanxia Liu et al.
Journal of pediatric endocrinology & metabolism : JPEM, 30(3), 349-353 (2017-03-12)
Background The DAX-1 gene is associated with X-linked adrenal hypoplasia congenita (AHC). Our objective was to compare the DAX-1 gene sequence of two male siblings exhibiting different clinical manifestations of AHC. Methods Clinical features were analyzed and laboratory data were

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